| Item type |
文献 / Documents(1) |
| 公開日 |
2023-01-31 |
| アクセス権 |
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|
アクセス権 |
open access |
| 資源タイプ |
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資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| 出版社版DOI |
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|
関連識別子 |
https://doi.org/10.1111/imcb.12546 |
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関連名称 |
10.1111/imcb.12546 |
| 出版タイプ |
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|
出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| タイトル |
|
|
タイトル |
Development of organ-specific autoimmunity by dysregulated Aire expression |
| タイトル別表記 |
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その他のタイトル |
Dysregulated Aire expression and autoimmunity |
| 著者 |
西嶋, 仁
スギタ, ミズキ
ウメザワ, ナツカ
キムラ, ナオキ
ササキ, ヒロカズ
河野, 弘
西岡, 安彦
松本, 穣
尾矢, 剛志
常山, 幸一
森本, 純子
松本, 満
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| 抄録 |
|
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内容記述 |
Deficiency for AIRE/Aire in both humans and mice results in the development of organ-specific autoimmune disease. We tested whether augmented and/or dysregulated AIRE/Aire expression might be also prone to the breakdown of self-tolerance. To define the effect of augmented Aire expression on the development of autoimmunity, antigen-specific clonal deletion and production of clonotypic regulatory T cells (Tregs) in the thymus were examined using mice expressing two additional copies of Aire in a heterozygous state (3xAire-knockin mice: 3xAire-KI). We found that both clonal deletion of autoreactive T cells and production of clonotypic Tregs in the thymus from 3xAire-KI were impaired in a T-cell receptor-transgenic system. Furthermore, 3xAire-KI females showed higher scores of experimental autoimmune encephalomyelitis induced by myelin oligodendrocyte glycoprotein than wild-type littermates, suggesting that augmented Aire expression exacerbates organ-specific autoimmunity under disease-prone conditions. In humans, we found that one patient with amyopathic dermatomyositis showed CD3–CD19– cells expressing AIRE in the peripheral blood before the treatment but not during the remission phase treated with immunosuppressive drugs. Thus, not only loss of function of AIRE/Aire but also augmented and/or dysregulated expression of AIRE/Aire should be considered for the pathogenesis of organ-specific autoimmunity. We suggest that further analyses should be pursued to establish a novel link between organ-specific autoimmune disease and dysregulated AIRE expression in clinical settings. |
| キーワード |
|
|
主題 |
Aire |
| キーワード |
|
|
主題 |
autoimmune disease |
| キーワード |
|
|
主題 |
EAE |
| キーワード |
|
|
主題 |
mTEC |
| キーワード |
|
|
主題 |
polymyositis |
| キーワード |
|
|
主題 |
self-tolerance |
| 書誌情報 |
en : Immunology & Cell Biology
巻 100,
号 5,
p. 371-377,
発行日 2022-03-21
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| 収録物ID |
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収録物識別子タイプ |
ISSN |
|
収録物識別子 |
14401711 |
| 収録物ID |
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収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA10678685 |
| 出版者 |
|
|
出版者 |
Australian and New Zealand Society for Immunology |
| 出版者 |
|
|
出版者 |
Wiley |
| 権利情報 |
|
|
権利情報 |
This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
| EID |
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|
識別子 |
387313 |
| 言語 |
|
|
言語 |
eng |
icb_100_5_371.pdf (781 KB)
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